Sickle Cell Intrahepatic Cholestasis: A Case Report

Abstract

Introduction: We present the case of a 47-year-old man with known sickle cell disease, who came to the hospital with worsening right hip pain and lethargy. The patient did not have any abdominal symptoms on presentation. Important past medical history included sickle cell disease, emphysema, avascular necrosis of the right femoral head, and splenectomy. The patient denied any fever or shortness of breath at the time of admission. Physical exam revealed drowsiness, but otherwise stable vital signs. Tenderness was elicited on palpation of the right hip and right upper quadrant of the abdomen. The rest of the examination was benign, including no focal neurological deficits. Laboratory examination revealed the following: WBC= 26000/cc, hemoglobin=6mg/dl, creatinine=5.1mg/dL, total bilirubin=46mg/dL, direct bilirubin=38.5mg/dL, AST=52U/L, ALT=16U/L, alkaline phosphatase=174U/L, and INR=1.6. Right upper quadrant ultrasound of the abdomen was unrevealing for any acute abnormality. Course of hospitalization: The patient was started on broad-spectrum antibiotics for possible hip infection. Exchange transfusions were initiated for acute sickle cell intrahepatic cholestasis (SCIC). Hip aspirate resulted negative for any signs of infection, and antibiotics were stopped. The patient underwent dialysis for acute kidney injury, but after a mild improvement for a short time, the patient developed respiratory failure and encephalopathy accompanied by further elevation of bilirubin and worsening renal failure. Exchange transfusion and dialysis were continued, but unfortunately, the patient went into multi-organ system failure and died. Liver disease in individuals with sickle cell disease can be a result of an acute sickling process within the hepatic sinusoids causing ischemia. SCIC is a rare disease with fewer than 20 reported adult cases thus far. Usual presentation is right upper quadrant pain with nausea, vomiting, fever, tender hepatomegaly followed by striking jaundice accompanied by renal impairment, bleeding diathesis, and encephalopathy. Hyperbilirubinemia has ranged from 30mg/dL to as high as 273mg/dL in various case reports. AST, ALT, and alkaline phosphatase levels are variable as well, ranging from normal to <1000 IU/L. The diagnosis is established by characteristic clinical and laboratory findings in the absence of a mechanical obstruction within the biliary tract. Older reported cases had very high mortality (>80%). This has improved with early institution of exchange transfusion, which is the mainstay of therapy along with supportive care. Nevertheless, favorable outcomes may not be achieved, as demonstrated by our case report, especially in patients who progress to multiorgan dysfunction state.